Dermatofibrosarcoma protuberans: an early non-protuberant phase of the tumour

A 40-year-old female patient presented with a flat scar like plaque on the upper chest. The patient's perception of subtle change in the lesion was of paramount importance in the decision to biopsy this lesion of innocuous clinical appearance. Histopathological findings were those of dermatofibrosarcoma protuberans. This case illustrates the early clinical features of the tumour and a recommendation for taking a biopsy of any scar-like plaque where there is no clear history of preceding trauma.     

Dermatofibrosarcoma protuberans and giant cell fibroblastoma exhibit CD99 positivity

According to most authors, dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) represent the adult and juvenile forms, respectively, of the same disease entity, as evidenced by similar morphology, an identical chromosomal translocation, and CD34 positivity. It has been shown that DFSP and nuchal-type fibroma (NTF) (which is also CD34-positive) are related lesions, and that there might possibly be a continuum between the two. In addition, NTF exhibits CD99 positivity. It was therefore, hypothesized that both DFSP and GCF would show similar immunopositivity for CD99. Archives of pathology at several institutions were searched for DFSP and GCF tissue blocks. A total of 29 DFSP and 5 GCF were analyzed by immunohistochemistry for expression of CD99. Twenty-three of 29 DFSP (79%) and 2 of 5 GCP (40%) expressed CD99. Comparison of CD99 and CD34 showed that the non-tumoral periphery of DFSP was less probable to be CD99 positive, but this finding was not statistically significant.     

隆突性皮肤纤维肉瘤1例

报告1例隆突性皮肤纤维肉瘤,患者女,36岁,因右颞部红色结节1年就诊。系统检查未发现异常。颞部结节经组织病理检查,诊断为隆突性皮肤纤维肉瘤,给予手术切除,术后半年随访无原位复发。     

Dermatofibrosarcoma protuberans: a tumour of nestin‐positive cutaneous mesenchymal stem cells?

Background There is an increasing body of evidence suggesting that malignancies arise from mutated stem cells, which has led to the formulation of the cancer stem cell hypothesis. It has also been suggested that cutaneous malignancies originate from a mutated stem cell. To date, mesenchymal tumours of the skin have not been the focus of the cancer stem cell hypothesis. A population of mesenchymal stem cells has recently been identified in the dermal compartment of the skin. These proposed stem cells are positive for the neuroepithelial stem cell marker nestin. Objectives  To describe the expression pattern of nestin, a neuroepithelial stem cell protein, in dermatofibrosarcoma protuberans (DFSP). Methods Immunohistochemical evaluation of DFSP with a monoclonal antibody against nestin was performed using standard techniques. For comparison we also analysed dermatofibromas (DF). In addition, we used antibodies against CD34 and Factor XIIIa; the proliferation marker Ki67 was also used. Results Strong immunoreactivity for nestin was found in DFSP whereas all DF cases were nestin‐negative. Conclusions We propose that DFSP may represent a clonal expansion of a nestin‐positive mesenchymal stem cell which would put this tumour in line with other neoplasms for which the cancer stem cell hypothesis was formulated. We suggest the use of nestin as an additional marker for DFSP, especially in cases of negative immunoreactivity for CD34. Nestin may also be employed for margin evaluation of DFSP in micrographic (Mohs) surgery.     

额部隆突性皮肤纤维肉瘤一例

患者男,56岁,因左前额部肿块1年余入院.患者20年前左前额部因黄豆大小肿块,曾行切除术.1年前肿块复发并增大,无明显自觉症状来我院就诊.皮肤科检查:左前额部见3 cm×2 cm×1 cm大小的肿块,呈淡红色半球形隆起于皮面,表面皮肤光滑,紧张,质地较硬,与皮肤粘连,但与深筋膜无明显粘连,无触压痛.肿块周围可见明显凹陷区.皮损组织病理:真皮中下层及脂肪内可见大量梭形细胞增生,细胞轻度异形,呈编织状排列.免疫组化染色:CD34(+),波形蛋白(+),CK(-),S-100(-),上皮膜抗原(-).诊断为隆突性皮肤纤维肉瘤.切除肿瘤并行游离全厚皮片移植,术后皮片生长良好,效果满意.     

儿童下肢隆突性皮肤纤维肉瘤一例

患儿女,6岁,因左小腿外侧结节2年就诊。患儿家长2年前发现患儿左小腿外侧出现1个淡红色绿豆大小皮肤结节,无明显痛痒感觉,结节逐渐增大。半年前结节直径增至1 cm左右,周边新发2个绿豆至黄豆大小淡红色结节,近半年结节体积逐渐变大……     

额部隆突性皮肤纤维肉瘤一例

A 56-year-old male was admitted to the hospital for a mass on the left forehead for more than one year. Twenty years prior to the presentation, the patient developed a broad bean-sized nodule on the left forehead, thereafter, an in situ resection was performed. One year prior to the presentation, the lesion recurred and gradually enlarged with no evident symptom. Physical examination showed a pink, painless and indurated mass measuring 3 cm × 2 cm × 1 cm on the left forehead. The lesion appeared as a hemispheroid-shaped elevation, had a smooth and tense surface, and was adherent to skin but not to deep fascia. There was an obvious depression around the mass. Histopathological examination revealed an evident proliferation of spindle cells with a braid-like distribution in the mid- and lower dermis. Mild atypia was observed in these cells.Immunohistochemistry revealed that the tumor cells were positive for CD34 and vimentin, but negative for CK, S-100 and epithelial membrane antigen. A diagnosis of dermatofibrosarcoma protuberans was made. The lesion was resected followed by a free full thickness skin graft. After operation, the flap survived well, and a satisfactory outcome was achieved.     

误诊为脂肪瘤的隆突性皮肤纤维肉瘤一例

患者,男,45岁.右侧胸部肿物6年,肿块位于皮下,质软,无疼痛不适.6年前B超检查示脂肪瘤可能性大,未予特殊处理.本次就诊行病理检查结果示软组织来源的梭形细胞肿瘤.诊断为隆突性皮肤纤维肉瘤,行肿瘤扩大根治术.     

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: a case report

报告1例伴纤维肉瘤转化的隆突性皮肤纤维肉瘤.患者男,32岁.右侧腰背部结节3年余.皮肤科检查:右侧腰部见一直径4～5 cm类圆形结节,触之较硬,无压痛,表面可见扩张的血管及手术瘢痕.组织病理见真皮及皮下有增生梭形细胞形成的肿瘤细胞团块,肿瘤内部分区域梭形细胞呈特征性席纹状排列,部分区域梭形细胞呈束状或鱼骨状排列,束状或鱼骨状排列区内梭形细胞可见异形核及核分裂象.免疫组化染色示:真皮及皮下的梭形细胞CD34(+)、波形蛋白(vimentin)(+)、Ki-67(+ >20%)、S-100蛋白(-)、平滑肌肌动蛋白(SMA)(-)、上皮膜抗原(EMA)(-)和Ⅷa(-).诊断为:伴纤维肉瘤转化的隆突性皮肤纤维肉瘤.     

Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases

AIM: To review the dinical and histological data of 20 cases of dermatofibrosarcoma protuberans presenting at two dermatology centres in Lisbon from 1978 to 1998. PATIENTS AND METHODS: The 20 subjects comprised nine males and 11 females ranging in age from 25 to 79 years, with highest frequency of subjects in the 30-50 year olds. We reviewed the clinical features, histopathological aspects, including morphologic variants and immunohistochemical studies. RESULTS: Median age at diagnosis was 51 years and the trunk was the most frequent location. The characteristic histologic storiform pattern was seen in all cases. Three subjects presented fibrosarcomatous areas, one with myoid differentiation and another with multinucleated giant cells. Immunohistochemical stains revealed CD34 expression in the 18 specimens tested, FXIIIa was negative, and these two antigens proved important for the differential diagnosis of this neoplasm. Local wide excision was performed in 13 cases and seven patients underwent Moh's micrographic surgery. Follow-up ranged from 2 months to 17 years and three recurrences were recorded, two following classical surgery and one after Moh's surgery; there was no difference in the rate of local recurrence (15%) for the two kinds of treatment in our series.     

Dermatofibrosarcoma protuberans in a pediatric patient with ataxia telangiectasia syndrome

Ataxia telangiectasia (AT) is a rare autosomal recessive neurodegenerative disorder caused by a mutation in the ATM gene. An impaired immune response due to the gene mutation leads to an increased risk of infection and malignancy. We present a rare case of dermatofibrosarcoma protuberans arising in a patient with AT.     

Dermatofibrosarcoma protuberans with onset in early childhood: a case report

Dermatofibrosarcoma protuberans is a tumour that grows locally, infiltrating the subcutaneous tissue; it is uncommon in children. We report the case of a 27-year-old man who had had such a lesion from the age of 3 years, and it had enlarged during his lifetime. A wide local excision with margins of 3.0 cm was performed, but the lesion was not entirely removed. This is a new report of infantile dermatofibrosarcoma protuberans, and this case reflects the need for Mohs micrographic surgery.     

Dermatofibrosarcoma protuberans: a clinicopathological analysis of 10 cases in Asians

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous neoplasm that exhibits a marked tendency for recurrence after local excision. This case series aims to study the clinical, histological and immunohistochemical features of DFSP in Asians. Ten patients with DFSP diagnosed between 1992 and 2001 were reviewed. There were more women than men in a ratio of 4:1. There were six Chinese, two Malays, one Indian and one Eurasian. The mean age was 38 years. The duration of each lesion before excision varied from 6 months to 27 years. Fifty per cent of tumours occurred on the trunk. On histology, all the lesions were dermal-centred spindle cell tumours, extending to the subcutis, and exhibited the characteristic storiform pattern. One tumour also demonstrated fibrosarcomatous changes. Two tumours were of the rare pigmented variant (Bednar tumour). Immunohistochemistry with CD34 was positive in all cases, except the fibrosarcomatous area of one tumour, which was negative for CD34. For comparison, six cases of deep-penetrating dermatofibroma were stained for CD34 and all showed an absence of CD34 expression. Wide excision of the tumour was performed in all cases of DFSP. There was no recurrence after mean follow up of 6 years (range 2.25-9.5 years).     

CD34与FXIIIa在隆突性皮肤纤维肉瘤和皮肤纤维瘤中的表达

目的:检测CD34和FXIIIa(Factor XIIIa)在隆突性皮肤纤维肉瘤(DFSP)和皮肤纤维瘤(DF)中的表达。方法:应用免疫组化结合阳性肿瘤细胞半定量法检测7例DFSP和13例DF中CD34与FXIIIa的表达。结果:7例DFSP CD34呈强而弥慢性阳性反应(阳性细胞率为4.6±0.03);13例DF皆为阴性。13例DF呈不同程度的FXIIIa阳性表达(阳性细胞率为3.6±0.02);而DFSP全部为FXIIIa阴性。结论:CD34和FXIIIa可分别作为鉴别DFSP和DF的重要标记物。     

Dermatofibrosarcoma protuberans in an infant with a history of choroid plexus papilloma

We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor.     

Cellular digital fibromas: distinctive CD34-positive lesions that may mimic dermatofibrosarcoma protuberans

BACKGROUND: Digital fibromas are common benign acral tumors typically reported as angiofibromas (AFs) or acquired digital fibrokeratomas (ADFs). Cellular variants are not well recognized. METHODS: We collected 14 acral fibrocytic lesions showing a spindle cell morphology from our files, and evaluated CD34, Factor XIIIa, epithelial membrane antigen (EMA), and S100 protein staining of these lesions. We compared the histologic and immunohistochemical features of these cellular fibromas with five digital AFs, five ADFs, and five digital dermatofibromas. RESULTS: The 14 cellular digital fibromas showed intersecting fascicles of thin delicate bland spindle cells in the superficial reticular dermis with a fibrotic-to-slight myxoid stroma. The spindle cells in all cases stained strongly for CD34, and only scattered stromal cells stained for Factor XIIIa. Five tested cases were negative for EMA and S100 protein. The digital AFs, fibrokeratomas, and dermatofibromas stained predominately for Factor XIIIa, with no or minimal staining for CD34. CONCLUSIONS: These findings suggest that a subset of digital fibromas is characterized by a dense cellular proliferation of CD34-positive spindle cells. Awareness of this variant of digital fibroma and its staining pattern is critical in preventing misdiagnosis as dermatofibrosarcoma protuberans, particularly in superficial biopsies.     

皮肤纤维瘤和隆突性皮肤纤维肉瘤中XIIIα因子与CD34的不同表达

目的：探讨XIIIα因子(FXIIIα)和CD34在鉴别皮肤纤维瘤(DF)和隆突性皮肤纤维肉瘤(DFSP)中的应用价值。方法：应用免疫组化技术对11例DF和8例DFSP中FXIIIα与CD34的表达进行了观察。结果：11例DF中9例FXIIIα阳性，全部病例CD34均为阴性；8例DFSP FXIIIα均示阴性，CD34皆为阳性。结论：FXIIIα和CD34可作为鉴别DF和DFSP的重要标记物。     

Recurrent Dermatofibrosarcoma Protuberans with Myxoid and Fibrosarcomatous Changes Paralleled by Loss of CD34 Expression

A 38-year-old woman complained of recurrent nodules on her anterior chest wall. She noticed the first nodule at the age of 12 and has since undergone surgical removal a total of 31 times. Finally, wide resection of parts of the rib cartilage and sternum and chest wall reconstruction were required. Histopathological examination of a series of surgical specimens were reevaluated. Immunohistochemical staining of the tumors by anti-CD34 antibody provided a definite diagnosis of dermatofibrosarcoma protuberans (DFSP). A noteworthy result was that her DFSP did not always express CD34; the recurrent tumors with myxoid and fibrosarcomatous changes lost their CD34 expression.